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1.
Pediatr Nephrol ; 2019 Oct 13.
Artigo em Inglês | MEDLINE | ID: mdl-31606750

RESUMO

BACKGROUND: The ideal management of ureteropelvic junction obstruction (UPJO) remains debatable. This prospective case-control study aimed to investigate if urinary levels of Neutrophil Gelatinase-Associated Lipocalin (NGAL) and serum levels of cystatin C could distinguish surgical from non-surgical cases of UPJO and if they could detect earlier impairment of renal function. METHODS: Biomarkers were measured in the following age-matched groups: (a) 22 infants with surgical UPJO, at initial diagnosis and 12 months postoperatively (groups A1 and A2, respectively); (b) 19 infants with non-surgical UPJO (group B); and (c) 17 controls (group C). Based on serum cystatin C levels, estimated glomerular filtration rate (eGFR) was calculated. RESULTS: Urinary NGAL (uNGAL) was significantly higher in group A1 vs. group A2 (p = 0.02) and in group A1 vs. group C (p = 0.03), whereas there was no statistically significant difference between groups A2 and C (p = 0.77). Likewise, cystatin C levels were significantly higher in group A1 vs. group A2 and in group A1 vs. group C (p = 0.004 and p = 0.02, respectively), but no statistically significant difference between groups A2 and C (p = 0.82). uNGAL and serum cystatin C did not differ between groups B and A, nor did they differ between groups B and C. Cystatin C levels and eGFR of group A1 were significantly higher than those of group A2 and group C (p = 0.0001 and p = 0.02, respectively). CONCLUSION: It seems that NGAL and cystatin C are able to distinguish patients who were treated surgically from healthy controls, and their levels appear to improve significantly following surgery.

2.
Rheumatol Int ; 38(7): 1241-1250, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29845429

RESUMO

To assess longitudinally the course and outcome of juvenile idiopathic arthritis (JIA) in patients diagnosed and followed-up exclusively in the biologic era; also, to define possible predictors of the disease progression and need for early implementation of biologicals. Prospective and retrospective, monocentric cohort study of 120 JIA patients, diagnosed between 2001 and 2010, and followed-up for ≥ 4 years (median 8.04). Disease activity, cumulative articular/extra-articular damage and quality of life were evaluated by the assessment tools Juvenile Arthritis Disease Activity Score (JADAS71), Juvenile Arthritis Damage Index (JADI) and Childhood Health Assessment Questionnaire (CHAQ), respectively. Moreover, potential predictors of the disease progression and their relation to biologic therapy were investigated. High JADAS71 score (> 9) at diagnosis was indicative of progression to polyarticular course and the need for early introduction of biologic treatment. Other independent predictors of progression to polyarthritis, were: involvement of upper limb, hip and ankle within 6 months following JIA diagnosis and percentage of cumulative time with active disease > 35% within the first year. At the end of the study, both the median JADAS71 score and the Disability Index were significantly lower than the initial (p < 0.001) and remission off medication was achieved in 25% of the patients. Articular and extra-articular (only ocular) cumulative damage was demonstrated only in 5 and 7.5% of patients, respectively. Physical functional ability was found normal/mildly restricted in 93.3% and moderately restricted in 6.7% of the patients. We believe that these findings, fit in with a picture of JIA course and outcome under current conditions of objective "disease status" evaluation and of tightly controlled follow-up. Predictors emerged from our study could contribute to the identification of patients who will need early implementation of biologic treatment.

3.
J Immunol Res ; 2017: 1514294, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28900629

RESUMO

Sialic acid acetylesterase (SIAE) deficiency was suggested to lower the levels of ligands for sialic acid-binding immunoglobulin-like receptors, decreasing the threshold for B-cell activation. In humans, studies of rare heterozygous loss-of-function mutations in SIAE gene in common autoimmune diseases, including juvenile idiopathic arthritis (JIA), yielded inconsistent results. Considering the distinct pathogenesis of the two main subtypes of JIA, autoinflammatory systemic (sJIA) and autoimmune oligo/polyarticular (aJIA), and a predisposition to autoimmunity displayed by patients and families with primary antibody deficiencies (PADs), the aim of our study was to analyze whether SIAE rare variants are associated with both the phenotype of JIA and the autoimmunity risk in families with PADs. A cohort of 69 patients with JIA, 117 healthy children, 54 patients, and family members with PADs were enrolled in the study. Three novel SIAE variants (p.Q343P, p.Y495X, and c.1320+33T>C) were found only in patients with aJIA but interestingly also in their healthy relatives without autoimmunity, while none of PAD patients or their relatives carried SIAE defects. Our results show that SIAE rare variants are not causative of autoimmunity as single defects.


Assuntos
Acetilesterase/genética , Artrite Juvenil/genética , Variação Genética , Síndromes de Imunodeficiência/genética , Acetilesterase/metabolismo , Adolescente , Alelos , Artrite Juvenil/enzimologia , Artrite Juvenil/imunologia , Autoimunidade , Estudos de Casos e Controles , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Predisposição Genética para Doença , Genótipo , Humanos , Síndromes de Imunodeficiência/enzimologia , Masculino , Mutação , Polimorfismo de Nucleotídeo Único
4.
J Pediatr Hematol Oncol ; 39(6): 413-419, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28654461

RESUMO

Primary Evans syndrome (ES) is defined by the concurrent or sequential occurrence of immune thrombocytopenia and autoimmune hemolytic anemia in the absence of an underlying etiology. The syndrome is characterized by a chronic, relapsing, and potentially fatal course requiring long-term immunosuppressive therapy. Treatment of ES is hardly evidence-based. Corticosteroids are the mainstay of therapy. Rituximab has emerged as the most widely used second-line treatment, as it can safely achieve high response rates and postpone splenectomy. An increasing number of new genetic defects involving critical pathways of immune regulation identify specific disorders, which explain cases of ES previously reported as "idiopathic".


Assuntos
Anemia Hemolítica Autoimune/patologia , Anemia Hemolítica Autoimune/terapia , Trombocitopenia/patologia , Trombocitopenia/terapia , Corticosteroides/uso terapêutico , Anemia Hemolítica Autoimune/etiologia , Anemia Hemolítica Autoimune/mortalidade , Criança , Transplante de Células-Tronco Hematopoéticas , Humanos , Imunossupressão/métodos , Rituximab/uso terapêutico , Esplenectomia , Trombocitopenia/etiologia , Trombocitopenia/mortalidade
6.
J Allergy Clin Immunol ; 139(4): 1282-1292, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-27697500

RESUMO

BACKGROUND: X-linked hyper-IgM syndrome (XHIGM) is a primary immunodeficiency with high morbidity and mortality compared with those seen in healthy subjects. Hematopoietic cell transplantation (HCT) has been considered a curative therapy, but the procedure has inherent complications and might not be available for all patients. OBJECTIVES: We sought to collect data on the clinical presentation, treatment, and follow-up of a large sample of patients with XHIGM to (1) compare long-term overall survival and general well-being of patients treated with or without HCT along with clinical factors associated with mortality and (2) summarize clinical practice and risk factors in the subgroup of patients treated with HCT. METHODS: Physicians caring for patients with primary immunodeficiency diseases were identified through the Jeffrey Modell Foundation, United States Immunodeficiency Network, Latin American Society for Immunodeficiency, and Primary Immune Deficiency Treatment Consortium. Data were collected with a Research Electronic Data Capture Web application. Survival from time of diagnosis or transplantation was estimated by using the Kaplan-Meier method compared with log-rank tests and modeled by using proportional hazards regression. RESULTS: Twenty-eight clinical sites provided data on 189 patients given a diagnosis of XHIGM between 1964 and 2013; 176 had valid follow-up and vital status information. Sixty-seven (38%) patients received HCT. The average follow-up time was 8.5 ± 7.2 years (range, 0.1-36.2 years). No difference in overall survival was observed between patients treated with or without HCT (P = .671). However, risk associated with HCT decreased for diagnosis years 1987-1995; the hazard ratio was significantly less than 1 for diagnosis years 1995-1999. Liver disease was a significant predictor of overall survival (hazard ratio, 4.9; 95% confidence limits, 2.2-10.8; P < .001). Among survivors, those treated with HCT had higher median Karnofsky/Lansky scores than those treated without HCT (P < .001). Among patients receiving HCT, 27 (40%) had graft-versus-host disease, and most deaths occurred within 1 year of transplantation. CONCLUSION: No difference in survival was observed between patients treated with or without HCT across all diagnosis years (1964-2013). However, survivors treated with HCT experienced somewhat greater well-being, and hazards associated with HCT decreased, reaching levels of significantly less risk in the late 1990s. Among patients treated with HCT, treatment at an early age is associated with improved survival. Optimism remains guarded as additional evidence accumulates.


Assuntos
Transplante de Células-Tronco Hematopoéticas/mortalidade , Síndrome de Imunodeficiência com Hiper-IgM/mortalidade , Síndrome de Imunodeficiência com Hiper-IgM/terapia , Adolescente , Adulto , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Seguimentos , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Tempo , Adulto Jovem
7.
Asian Pac J Allergy Immunol ; 34(2): 159-65, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27007838

RESUMO

BACKGROUND: The Paediatric Allergic Rhinitis Quality of Life Questionnaire (Ped-AR-QoL) is the first tool developed for the assessment of health-related quality of life (QoL) in Greek children with allergic rhinitis (AR). OBJECTIVE: The aim of the current study was to validate the child and parent forms of the Ped-AR-QoL in children aged 6-14 years-old who suffered from AR and were followed in a pediatric allergy clinic. METHODS: The Ped-AR-QoL, which was completed by 112 children and their parents, was correlated to the generic QoL questionnaire (Disabkids), which is already valid in Greece for children with chronic disorders, as well as with expert opinions on the severity of disease. RESULTS: The Ped-AR-QoL child and parent forms had very good internal consistency (α values of 0.797 and 0.872, respectively), while there was a moderate positive correlation of the disease-specific questionnaire with most of the subscales of the generic questionnaire. There has been a statistically significant association between the Ped-AR-QoL and the expert perception of disease severity. CONCLUSIONS: The Ped-AR-QoL had very good reliability and convergent validity when compared with the generic Disabkids QoL. The significance of the association between the disease-specific questionnaire and the expert opinion is an important finding validating the questionnaire. The Ped-AR-QoL may become a helpful tool which can be used in everyday clinical practice by clinicians and it may also be used for assessing therapeutic interventions in clinical trials.


Assuntos
Qualidade de Vida , Rinite Alérgica/diagnóstico , Inquéritos e Questionários , Adolescente , Fatores Etários , Criança , Efeitos Psicossociais da Doença , Feminino , Grécia , Humanos , Masculino , Pais/psicologia , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Rinite Alérgica/psicologia , Índice de Gravidade de Doença
8.
Ann Gastroenterol ; 28(4): 452-6, 2015 Oct-Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26423109

RESUMO

BACKGROUND: Ghrelin and obestatin are peptides of the gut-brain axis affecting appetite and gastrointestinal motility. METHODS: We conducted a cross-sectional, case-control study to determine pre- and postprandial serum levels of total ghrelin and obestatin along with gastric emptying scintigraphy in children with symptoms suggestive of delayed gastric emptying time (GET), not attributable to any identifiable cause. RESULTS: Twenty children with symptoms suggestive of delayed GET, of whom 9 had delayed GET, and 20 age-matched healthy children were enrolled. Preprandial ghrelin and obestatin were higher compared to controls (GHR mean level in patients and controls: 1162 pg/mL and 401 pg/mL respectively; P<0.05; OB mean level in patients and controls: 417 pg/mL and 325 pg/mL respectively; not statistically significant). Postprandial ghrelin was significantly decreased in the subgroup of patients with delayed GET (GHR mean level in children with normal and prolonged GET: 1237 pg/mL and 584 pg/mL respectively; P<0.05). CONCLUSION: Obestatin and ghrelin were deranged in children with symptoms indicative of delayed GET of unexplained etiology. Gastric emptying was prolonged in almost half of the patients thus gastric emptying scintigraphy should be considered in the investigation of children with such symptomatology.

9.
Int Arch Allergy Immunol ; 164(4): 326-32, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-25277223

RESUMO

BACKGROUND: No published data presently exist concerning hereditary angioedema (HAE) in Greece. The aim of this study was to present the results from patients recorded by the Greek Hereditary Angioedema Registry over the last 3 years (July 2010 to June 2013). METHODS: A systematic recording of HAE cases was undertaken following a physician awareness campaign and confirmation of diagnosis. A questionnaire was also used for the assessment of key parameters of the patients' disease-specific quality of life. RESULTS: One hundred and sixteen patients from 41 non-related families were recorded. There were 33 (80.5%) families with type I HAE, 7 (17%) with type II HAE and 1 (2.5%) with non-C1 inhibitor (C1-INH), non-FXII HAE. Two further non-C1-INH, non-FXII HAE sporadic cases were recorded. An investigation of non-symptomatic family members revealed another 6 asymptomatic individuals with C1-INH deficiency. The average delay in diagnosis was 16.5 years and the incidence of death in the families of patients was 1 for every 2 families. The use of newer therapeutic agents seems to fall significantly short of the existing needs. HAE was found to affect the quality of life slightly in 14%, greatly in 63% and significantly in 23% of the patients. CONCLUSION: Until recently, there has been a significant degree of underdiagnosis of HAE in Greece. Very low compliance with the provisions of the applicable international guidelines and consensus positions, with adverse consequences on the patients' quality of life, was also observed. The centralized model we used to uncover the patients could be effective in other countries presenting with comparable disease characteristics.


Assuntos
Angioedemas Hereditários/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Angioedemas Hereditários/diagnóstico , Angioedemas Hereditários/tratamento farmacológico , Anti-Inflamatórios não Esteroides/uso terapêutico , Bradicinina/análogos & derivados , Bradicinina/uso terapêutico , Criança , Pré-Escolar , Danazol/uso terapêutico , Antagonistas de Estrogênios/uso terapêutico , Feminino , Seguimentos , Grécia/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Qualidade de Vida , Sistema de Registros , Inquéritos e Questionários , Adulto Jovem
10.
J Allergy Clin Immunol ; 134(1): 116-26, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24582312

RESUMO

BACKGROUND: Common variable immunodeficiency (CVID) is an antibody deficiency with an equal sex distribution and a high variability in clinical presentation. The main features include respiratory tract infections and their associated complications, enteropathy, autoimmunity, and lymphoproliferative disorders. OBJECTIVE: This study analyzes the clinical presentation, association between clinical features, and differences and effects of immunoglobulin treatment in Europe. METHODS: Data on 2212 patients with CVID from 28 medical centers contributing to the European Society for Immunodeficiencies Database were analyzed retrospectively. RESULTS: Early disease onset (<10 years) was very frequent in our cohort (33.7%), especially in male subjects (39.8%). Male subjects with early-onset CVID were more prone to pneumonia and less prone to other complications suggesting a distinct disease entity. The diagnostic delay of CVID ranges between 4 and 5 years in many countries and is particularly high in subjects with early-onset CVID. Enteropathy, autoimmunity, granulomas, and splenomegaly formed a set of interrelated features, whereas bronchiectasis was not associated with any other clinical feature. Patient survival in this cohort was associated with age at onset and age at diagnosis only. There were different treatment strategies in Europe, with considerable differences in immunoglobulin dosing, ranging from 130 up to 750 mg/kg/mo. Patients with very low trough levels of less than 4 g/L had poor clinical outcomes, whereas higher trough levels were associated with a reduced frequency of serious bacterial infections. CONCLUSION: Patients with CVID are being managed differently throughout Europe, affecting various outcome measures. Clinically, CVID is a truly variable antibody deficiency syndrome.


Assuntos
Imunodeficiência de Variável Comum/complicações , Transtornos Linfoproliferativos/complicações , Pneumonia/complicações , Adolescente , Adulto , Idade de Início , Autoimunidade , Bronquiectasia/patologia , Criança , Pré-Escolar , Imunodeficiência de Variável Comum/tratamento farmacológico , Imunodeficiência de Variável Comum/imunologia , Imunodeficiência de Variável Comum/mortalidade , Diagnóstico Tardio , Europa (Continente) , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Transtornos Linfoproliferativos/tratamento farmacológico , Transtornos Linfoproliferativos/imunologia , Transtornos Linfoproliferativos/mortalidade , Masculino , Pneumonia/tratamento farmacológico , Pneumonia/imunologia , Pneumonia/mortalidade , Estudos Retrospectivos , Esplenomegalia/patologia , Análise de Sobrevida
11.
Pediatr Blood Cancer ; 61(6): 1017-22, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24453114

RESUMO

BACKGROUND: We aimed to investigate whether the presence of mannose binding lectin (MBL2), ficolin 2 (FCN2) polymorphisms or the combined deficiency significantly influence the risk and subsequently the frequency of chemotherapy-induced bacterial infections in children with B acute lymphoblastic leukemia (B-ALL). PROCEDURE: MBL2 polymorphisms for exon 1 and FCN2 polymorphisms for promoter regions -986, -602, -557, -64, -4 and exon 8 regions +6,359, +6,424 were determined in children with B-ALL. FCN2 haplotype was determined by gene sequencing. Number and duration of FN episodes as well as number of bacterial infections were recorded during induction chemotherapy. RESULTS: Forty-four children with B-ALL (median age 4.3 years, 65.9% males) suffered from 142 FN episodes and 92 bacterial infections (40.2% Gram positive and 59.8% Gram negative). MBL2 low-risk genotype was found in 59.1%, medium-risk in 31.8% and high-risk in 9%. FCN2 low-risk haplotypes were detected in 38.2%, medium-risk in 44.1% and high-risk in 17.6%. MBL2 genotype and FCN2 haplotype were not associated with increased frequency of FN episodes. MBL2 medium/high-risk genotype and FCN2 medium/high-risk haplotype were associated with prolonged duration of FN (P = 0.007 and P = 0.001, respectively) and increased number of bacterial infections (P = 0.001 and P = 0.002, respectively). The combined MBL2/FCN2 medium/high-risk genotype was associated with an increased number of bacterial infections (P = 0.001). CONCLUSIONS: MBL2 and FCN2 single or combined deficiencies are associated with increased duration of FN episodes as well as increased number of bacterial infections in children with B-ALL suggesting a prognostic role of these genes.


Assuntos
Infecções Bacterianas/genética , Neutropenia Febril/genética , Lectinas/fisiologia , Lectina de Ligação a Manose/fisiologia , Polimorfismo Genético , Leucemia-Linfoma Linfoblástico de Células Precursoras B/genética , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Infecções Bacterianas/etiologia , Criança , Pré-Escolar , Códon/genética , Éxons/genética , Neutropenia Febril/induzido quimicamente , Feminino , Predisposição Genética para Doença , Genótipo , Haplótipos , Humanos , Imunidade Inata , Hospedeiro Imunocomprometido , Lactente , Lectinas/deficiência , Lectinas/genética , Masculino , Lectina de Ligação a Manose/deficiência , Lectina de Ligação a Manose/genética , Lectina de Ligação a Manose/imunologia , Erros Inatos do Metabolismo/complicações , Erros Inatos do Metabolismo/imunologia , Leucemia-Linfoma Linfoblástico de Células Precursoras B/complicações , Risco
12.
Am J Infect Control ; 40(1): 11-5, 2012 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-21856046

RESUMO

BACKGROUND: Serratia marcescens can cause health care-associated infections. We herewith report the investigation and control of an outbreak of S marcescens bloodstream infections (BSI) in a general pediatric department. METHODS: From April to May 2009, temporally related cases of S marcescens BSI occurred in a 40-bed general pediatric department of a tertiary care hospital. An outbreak investigation including case identification, review of medical records, environmental cultures, patients' surveillance cultures, personnel hand cultures, pulsed-field gel electrophoresis, and a case-control study were conducted. Controls were patients without S marcescens BSI but hospitalized in the department for at least 48 hours during the outbreak. Enhanced infection control measures were immediately implemented by the Infection Control Committee. RESULTS: During the study period, 4 patients developed BSI because of a S marcescens strain demonstrating the same antimicrobial susceptibility pattern as well as the same molecular profile. Patients' surveillance cultures and personnel hand cultures were negative. In 1 case-patient, S marcescens grew from cultures of intravenous infusion systems. In the case-control study performed, there were no differences in demographics, intravenously administered medications, or place of hospital stay. Case patients had changes in vascular access significantly more frequently than controls. No S marcescens infections occurred in the department during the 18 months following implementation of the enhanced infection control measures. CONCLUSION: Prompt recognition and strict adherence to infection control measures are of paramount importance in combating an outbreak of S marcescens bloodstream infection.


Assuntos
Bacteriemia/epidemiologia , Infecção Hospitalar/epidemiologia , Surtos de Doenças , Infecções por Serratia/epidemiologia , Serratia marcescens/isolamento & purificação , Bacteriemia/microbiologia , Estudos de Casos e Controles , Criança , Pré-Escolar , Infecção Hospitalar/microbiologia , Eletroforese em Gel de Campo Pulsado , Feminino , Genótipo , Grécia/epidemiologia , Hospitais Pediátricos , Humanos , Lactente , Controle de Infecções/métodos , Masculino , Epidemiologia Molecular , Tipagem Molecular , Infecções por Serratia/microbiologia , Serratia marcescens/classificação , Serratia marcescens/genética
13.
J Pediatr Hematol Oncol ; 32(6): e227-9, 2010 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-20539239

RESUMO

SUMMARY: The researchers describe the case of a earlier healthy 3-year-old boy, who developed immune thrombocytopenic purpura (ITP) 26 days after immunization with the second dose of seasonal influenza vaccine. He recovered quickly and uneventfully within 2 days after receiving a single dose of intravenous immunoglobulin. Review of the medical literature showed that symptomatic thrombocytopenia occurs in a substantial number of children and adults who require hospitalization for complicated natural influenza infection, particularly avian influenza. In contrast, it is exceptionally rare after influenza immunization, as only few case reports describe such an association in adults but not in children. As the risk of thrombocytopenia after natural influenza seems to be much higher than after immunization, annual influenza vaccination is advised for patients with personal history of ITP who at risk of influenza-related complications owing to underlying medical problems.


Assuntos
Vacinas contra Influenza/efeitos adversos , Púrpura Trombocitopênica Idiopática/etiologia , Púrpura Trombocitopênica Idiopática/fisiopatologia , Pré-Escolar , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , Púrpura Trombocitopênica Idiopática/tratamento farmacológico
14.
Vaccine ; 28(31): 5109-13, 2010 Jul 12.
Artigo em Inglês | MEDLINE | ID: mdl-20470802

RESUMO

Our aim was to study the effect of anti-TNF treatment on immunogenicity and safety of the 7-valent conjugate pneumococcal vaccine in children with juvenile idiopathic arthritis. Thirty-one children (mean age:12.9+/-4.6 years) treated with anti-TNFs plus Disease Modifying Anti-Rheumatic Drugs (DMARDs) and 32 age-matched children treated only with DMARDs were vaccinated with two doses of PCV7. After the first vaccine dose geometric mean titers (GMTs) were significantly increased for all vaccine serotypes (p<0.0001) in both groups and were found to be protective (>0.35microg/ml) in 87-100% of all children, depending on the serotype. Children receiving anti-TNFs achieved a significantly lower GMTs against serotypes 4, 14 and 23F (p<0.05). A >or=4-fold increase of the baseline titers to >or=5 vaccine serotypes was observed in 50% and 75% of the anti-TNF and control patients, respectively (p=0.0697). No patient developed vaccine-associated serious adverse events or disease flares.


Assuntos
Antirreumáticos/uso terapêutico , Artrite Juvenil/tratamento farmacológico , Vacinas Pneumocócicas/imunologia , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adolescente , Anticorpos Antibacterianos/sangue , Artrite Juvenil/imunologia , Criança , Pré-Escolar , Ciclosporina/uso terapêutico , Feminino , Vacina Pneumocócica Conjugada Heptavalente , Humanos , Masculino , Metotrexato/uso terapêutico , Vacinas Pneumocócicas/efeitos adversos , Prednisona/uso terapêutico , Vacinas Conjugadas/efeitos adversos , Vacinas Conjugadas/imunologia
16.
J Pediatr ; 156(4): 623-8, 2010 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-20097358

RESUMO

OBJECTIVE: To determine the incidence of immune thrombocytopenic purpura (ITP) after measles-mumps-rubella (MMR) immunization compared with natural measles and rubella, its clinical course and outcome, and the risk of recurrence after repeat MMR vaccination. STUDY DESIGN: We performed a systematic review of the Ovid MEDLINE (1950 to present) bibliographic database. We selected studies that reported cases of thrombocytopenia in a known number of children who were immunized with MMR vaccine before development of ITP. We also extracted data from the same and other studies regarding bleeding manifestations and the resolution of MMR-associated thrombocytopenia or thrombocytopenic purpura within 6 months. Finally, we studied the risk of ITP recurrence after MMR immunization or reimmunization. RESULTS: On the basis of 12 studies, the incidence of MMR-associated ITP ranged from 0.087 to 4 (median 2.6) cases per 100,000 vaccine doses. Severe bleeding manifestations were rare, and MMR-associated thrombocytopenia resolved within 6 months from diagnosis in 93% of the children. MMR vaccination of unimmunized patients with ITP and revaccination of patients with prior ITP did not lead to recurrence of thrombocytopenia. CONCLUSIONS: MMR-associated ITP is rare, self-limited, and non-life threatening, and susceptible children with ITP should be immunized with MMR at the recommended ages.


Assuntos
Vacina contra Sarampo-Caxumba-Rubéola/efeitos adversos , Guias de Prática Clínica como Assunto/normas , Púrpura Trombocitopênica/induzido quimicamente , Vacinação/efeitos adversos , Humanos , Incidência , Púrpura Trombocitopênica/epidemiologia , Fatores de Risco
18.
Hematology ; 12(6): 549-53, 2007 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-17852457

RESUMO

BACKGROUND: Pearson syndrome is a rare mitochondrial disorder characterized by sideroblastic anemia, liver disease, renal tubulopathy and exocrine pancreas deficiency. OBSERVATIONS: We describe a female infant suffering from anemia since birth who gradually developed the complete picture of Pearson syndrome by 13 months. Iron overload was disproportionate to blood transfusions. The patient was heterozygous for HFE gene C282Y mutation (type I hemochromatosis). After an initial response to deferoxamine she presented with cutaneous zygomycosis and died after metabolic derangement and Pneumocystis jiroveci pneumonia. CONCLUSION: This is the second case of a Pearson syndrome individual who was also heterozygous for HFE gene mutation C282Y published. It is also the second case report of a Pearson patient suffering from severe iron overload and liver disease that responded to therapy with deferoxamine.


Assuntos
Anemia Sideroblástica/complicações , Hemocromatose/complicações , Antígenos de Histocompatibilidade Classe I/genética , Proteínas de Membrana/genética , Doenças Mitocondriais/complicações , Mutação de Sentido Incorreto , Desferroxamina/uso terapêutico , Evolução Fatal , Feminino , Proteína da Hemocromatose , Heterozigoto , Humanos , Lactente , Sobrecarga de Ferro , Hepatopatias , Síndrome
19.
Am J Perinatol ; 24(2): 127-35, 2007 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-17304423

RESUMO

A prospective study was conducted to determine risk factors for fungal colonization, drug susceptibility, and association with invasive fungal infections (IFIs) in a neonatal unit. On admission and weekly thereafter, surveillance fungal cultures were taken from mouth, rectum, and trachea of neonates with expected stays of > 1 week. Fungal colonization was detected in 72 (12.1%) of 593 neonates during 12 months. CANDIDA ALBICANS was isolated from 42% of colonized neonates. Although early colonization (age 1.3 +/- 0.2 days) was found in 2.5% of the neonates, late colonization (age 17.6 +/- 1.4 days) was noted in 14.2% of neonates hospitalized for > 5 days. Neonates born vaginally were at higher risk for early colonization than those delivered after cesarean section ( P = 0.01). By multivariate logistic regression, very low birthweight was the only independent risk factor for late colonization. Ten IFIs (nine candidemias) were diagnosed, yielding a rate of 1.1%. These episodes occurred in 6.9% of colonized neonates, compared with 0.76% of noncolonized neonates ( P = 0.002). C. ALBICANS was susceptible to azoles, but some non- ALBICANS CANDIDA spp. exhibited decreased susceptibility to these drugs.


Assuntos
Infecção Hospitalar/epidemiologia , Unidades de Terapia Intensiva Neonatal , Micoses/epidemiologia , Candidíase/epidemiologia , Cesárea , Feminino , Humanos , Recém-Nascido , Masculino , Boca/microbiologia , Estudos Prospectivos , Reto/microbiologia , Fatores de Risco , Fatores de Tempo , Traqueia/microbiologia
20.
Infect Control Hosp Epidemiol ; 28(1): 60-7, 2007 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-17230389

RESUMO

OBJECTIVE: To evaluate filamentous fungi with respect to environmental load and potential drug resistance in a tertiary care teaching hospital. DESIGN: Monthly survey in 2 buildings of the hospital during a 12-month period. SETTING: Hippokration Hospital in Thessaloniki, Greece. METHODS: Air, surface, and tap water sampling was performed in 4 departments with high-risk patients. As sampling sites, the solid-organ transplantation department and the hematology department (in the older building) and the pediatric oncology department and the pediatric intensive care unit (in the newer building) were selected. RESULTS: From January to May of 2000, the fungal load in air (FLA) was low, ranging from 0 to 12 colony-forming units (cfu) per m(3) in both buildings. During the summer months, when high temperature and humidity predominate, the FLA increased to 4-56 cfu/m(3). The fungi commonly recovered from culture of air specimens were Aspergillus niger (25.9%), Aspergillus flavus (17.7%), and Aspergillus fumigatus (12.4%). Non-Aspergillus filamentous fungi, such as Zygomycetes and Dematiaceous species, were also recovered. The pediatric intensive care unit had the lowest mean FLA (7.7 cfu/m(3)), compared with the pediatric oncology department (8.7 cfu/m(3)), the solid-organ transplantation department (16.1 cfu/m(3)), and the hematology department (22.6 cfu/m(3)). Environmental surfaces were swabbed, and 62.7% of the swab samples cultured yielded filamentous fungi similar to the fungi recovered from air but with low numbers of colony-forming units. Despite vigorous sampling, culture of tap water yielded no fungi. The increase in FLA observed during the summer coincided with renovation in the building that housed the solid-organ transplantation and hematology departments. All 54 Aspergillus air isolates randomly selected exhibited relatively low minimum inhibitory or effective concentrations for amphotericin B, itraconazole, voriconazole, posaconazole, micafungin, and anidulafungin. CONCLUSION: Air and surface fungal loads may vary in different departments of the same hospital, especially during months when the temperature and humidity are high. Environmental Aspergillus isolates are characterized by lack of resistance to clinically important antifungal agents.


Assuntos
Antifúngicos/farmacologia , Monitoramento Ambiental/métodos , Fungos/efeitos dos fármacos , Hospitais Universitários , Microbiologia do Ar , Aspergillus/classificação , Aspergillus/isolamento & purificação , Água Doce/microbiologia , Fungos/classificação , Fungos/isolamento & purificação , Grécia , Arquitetura Hospitalar , Humanos , Testes de Sensibilidade Microbiana , Abastecimento de Água
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